Giant Retroperitoneal Liposarcoma with Mixed Histological Pattern: A Rare Presentation and Literature Review

Background

Retroperitoneal liposarcoma is a rare tumor that may grow to a considerable size before causing clinical symptoms. Mixed-type retroperitoneal liposarcoma is a very rare clinical entity.

Methods

We herein describe a rare case of a 54-year-old female who was diagnosed with a giant retroperitoneal liposarcoma arising from the right perinephric space.

Results

Radical nephrectomy and right salpingo-oophorectomy were nessecary to achieve complete tumor excision. Histological examination revealed a mixed-type liposarcoma consisting of well-differentiated and pleomorphic elements and deep invasion into the renal parenchyma, which is quite infrequent. The patient was well and disease-free 12 months after surgery.

Conclusions

Mixed-type retroperitoneal liposarcoma is a rare tumor. Aggressive surgical resection of the tumor together with adjacent structures, if necessary, is the mainstay of treatment.     

Sacral chordoma: management of a rare disease in a tertiary hospital

Introduction

Chordoma is a rare malignant bone tumor. The outcome depends on the adequate surgical treatment with complete excision of the tumor with microscopic clear margins.

Materials and methods

We performed a retrospective review of 13 cases of chordomas operated in our center between 1988 and 2009.

Results

All cases were treated with wide excision. Inadequate margins were found in seven patients. There were complications in ten patients and long-term sequels in 11 patients. Nine had recurrence within the first 2 years, six of which had inadequate margins. Five had metastatic disease, three of them with inadequate margins. During the follow-up, four patients died. In a posterior review, three patients survive and all of them had clear margins.

Conclusions

In our experience, the best treatment is complete excision of the tumor with clear microscopic margins. When this is not possible, local recurrences increase and survival rate decreases.     

Skin malignancies in Calabar, Southern Nigeria

Background

Skin cancers are among the most common form of cancer. In Africa, several reports reveal squamous cell carcinoma (SCC) as the commonest skin malignancy. In Africa, including regions of the same country, there are significant differences in the pattern of skin malignancy. This study sought to evaluate the pattern, risk factors and outcome of management of skin malignancy in our setting.

Methods

Patients with histologic diagnoses of skin malignancy from January 2007 and December 2008 who presented to the University of Calabar Teaching Hospital were prospectively studied.

Results

Forty-five patients presented with skin malignancy during the study period. The ages of the patients ranged from 5 and 75 years (mean: 46.6 years). The commonest skin malignancy was SCC in 19 (42.2%) patients while Kaposi sarcoma (KS) ranked second 15 (33.3%). Six patients presented with melanoma, two with metastatic carcinoma, basal cell carcinoma (BCC) two patients and a patient with dermatofibrosarcoma protuberans (DFSP). The lower limb was the commonest site involved in 25 (55.6%) patients, while the head and neck 11 (24.4%) ranked second.

Conclusion

SCC was the commonest skin malignancy. Non-solar factors of chronic ulcers in SCC, immunosuppression in KS, were identified as possible risk factors in some malignancies. This is at variance with the experience in Caucasians where solar radiation is the predominant risk factor. However, albinism and solar radiation were identified as risk factors in keeping with the Caucasians. Health education strategies on prevention, early presentation, and surgical evaluation of chronic ulcers would improve outcome.     

Carcinome adénoïde kystique du sein: à propos d’un cas

Introduction

Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm, accounting for less than 1% of breast carcinomas. The aim of our study is to describe the epidemiological and clinicopathological characteristics, the treatment and the prognosis of this type of breast tumor.

Case report

We report a new observation occurring in a 53-year-old female patient, who consulted for a left breast nodule. The ultrasound and mammography showed that it was a well-limited mass. Diagnosis was based on a histopathological exam completed by histochimical techniques. Estrogen and progesterone receptors were positive. The patient was treated by lumpectomy with adjuvant radiotherapy and hormonal therapy.

Discussion

ACC of the breast has a favorable prognosis. The diagnosis is made by histological examination with histochemical or immunohistochemical techniques. The treatment is not well established. It consists of lumpectomy with radiation or mastectomy. Lymph node involvement or distant metastases seldom occur.     

Indikationen zur Strahlentherapie beim lokal rezidivierten Mammakarzinom

Background

Radiotherapy is an important option in the treatment of locally recurrent breast cancer. In contrast to the first-line therapy of breast cancer, randomized trials on locally recurrent disease are rare.

Aim

The goal of this paper is to describe the current indications and radiotherapy options for locally recurrent breast cancer.

Methods

After performing a selective literature review, clinical trial data were compared with our own results.

Results

For isolated in-breast tumor recurrence after primary breast conserving surgery (BCS), salvage mastectomy is standard therapy. However, in individual cases, re-BCS is also a possible option. After re-BCS, partial breast radiation can result in improved local control. However, randomized studies comparing this option with the results obtained after salvage mastectomy are lacking. Complete surgical removal is the first treatment option for chest wall recurrence. If during primary treatment no irradiation was applied, then extended field irradiation of the whole chest wall is indicated. In cases with high risk of further relapse or with inoperable recurrence, re-irradiation is possible. A combination with hyperthermia or chemotherapy can be used for radiation sensitization.

Conclusion

Results of second BCS with additional partial breast irradiation are promising. However, larger randomized studies are required. In the treatment of a chest wall recurrence, irradiation can improve local control. Re-irradiation is possible, but must always be made based on the individual case.     

Primary Intra-abdominal Malignant Fibrous Histiocytoma: A Highly Aggressive Tumor

Background and Purpose

Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma of late adult life occurring predominantly in the extremities. Primary intra-abdominal MFH is a very rare occurrence. The aim of this study is to describe a very rare case of an intra-abdominal MFH with a highly aggressive clinical course.

Methods

A 67-year-old male was referred to our department with a 2-week history of dull lower abdominal pain and a gradually enlarging right lower abdominal mass, which he first noticed 2 months prior to admission. Computed tomography (CT) scan demonstrated a mass in the right iliac fossa.

Results

On exploratory laparotomy, a tumor was found in the right iliac fossa attached to the parietal lateral peritoneum without any evidence of invasion into the adjacent structures. Complete excision of the tumor with clear margins was performed. Histological and immunohistochemical examinations showed a MFH. One month after surgery, while on adjuvant chemotherapy, the patient was readmitted with dyspnea and a slightly palpable mass in the area of the previous radical resection. CT scan revealed local tumor recurrence along with multiple pulmonary metastatic deposits. Unfortunately, despite treatment, the patient died of progressive disease 5 weeks later.

Conclusions

Primary intra-abdominal MFH is a very rare but aggressive malignancy with a high tendency of local recurrence and metastatic spread. Early detection and complete surgical excision with clear margins is the treatment of choice. In some cases, however, the tumor can exhibit a highly aggressive clinical course despite radical surgery and adjuvant therapy.     

Dermatofibrosarcoma protuberans: therapeutic challenges

Background

Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal neoplasm with proclivity for recurrence with little metastatic potential.

Methods

Patients with histologic diagnosis of DFSP seen during a ten year period (January 2000 to December 2009) at the University of Calabar Teaching Hospital Calabar, Nigeria were studied.

Results

In total, 11 patients (5 males and 6 females) whose ages ranged from 17–52 years (mean 31.7 years) accounted for 6.8% of skin malignancies. The commonest site was the trunk 6 (54.5%), the head and neck ranked second, 3 (27.3%) followed by the lower limb 2 (18.2%). Eight (73%) patients presented with recurrent lesions. Follow up was poor (mean 6.8 months).

Conclusion

DFSP is an uncommon tumour with most patients presenting with recurrent lesions. Follow up was poor; consequently, we advocate adjuvant radiotherapy and treatment with imatinib be included in patients treatment plan from the onset.     

Mammographic density is related to stroma and stromal proteoglycan expression

Background

Mammographic density and certain histological changes in breast tissues are both risk factors for breast cancer. However, the relationship between these factors remains uncertain. Previous studies have focused on the histology of the epithelial changes, even though breast stroma is the major tissue compartment by volume. We have previously identified lumican and decorin as abundant small leucine-rich proteoglycans in breast stroma that show altered expression after breast tumorigenesis. In this study we have examined breast biopsies for a relationship between mammographic density and stromal alterations.

Methods

We reviewed mammograms from women aged 50–69 years who had enrolled in a provincial mammography screening program and had undergone an excision biopsy for an abnormality that was subsequently diagnosed as benign or pre-invasive breast disease. The overall mammographic density was classified into density categories. All biopsy tissue sections were reviewed and tissue blocks from excision margins distant from the diagnostic lesion were selected. Histological composition was assessed in sections stained with haematoxylin and eosin, and the expression of lumican and decorin was assessed by immunohistochemistry; both were quantified by semi-quantitative scoring.

Results

Tissue sections corresponding to regions of high in comparison with low mammographic density showed no significant difference in the density of ductal and lobular units but showed significantly higher collagen density and extent of fibrosis. Similarly, the expression of lumican and decorin was significantly increased.

Conclusion

Alteration in stromal composition is correlated with increased mammographic density. Although epithelial changes define the eventual pathway for breast cancer development, mammographic density might correspond more directly to alterations in stromal composition.     

A case of dermatofibrosarcoma protuberans in the skin over the breast

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing but locally aggressive, fibrous tumor that has a high rate of local recurrence after surgical resection. This tumor occurs most commonly in the trunk and proximal extremities. In this report we present a case of dermatofibrosarcoma protuberans in the skin over the breast which was re-excised after pathological diagnosis, considering cosmetic aspects. Only three other cases of dermatofibrosarcoma protuberans of the anterior chest wall have been encountered in our hospital, and all of these cases were male. The presence of this tumor in the skin over the breast appears to be rare in females. Careful complete resection is recommended for this type of tumor.     

Profil moléculaire du carcinome micropapillaire invasif du sein

Background

Invasive micropapillary carcinoma of the breast is a rare and aggressive histological variant of breast carcinoma. The new molecular classification of breast cancers distinguishing five subtypes, which are characterized by distinct molecular and immunohistochemical features as well as the histological one.

Aims of study

Define the molecular characteristics of invasive micropapillary carcinoma of the breast.

Methods

This is a retrospective review, from 2002 to 2008, included 7 cases of invasive micropapillary carcinoma of the breast diagnosed at the Department of Pathology, Farhat Hached hospital, Sousse, Tunisia. The clinico-pathological features, treatment modalities as well as immunohistochemical features patient??s outcome were recorded.

Results

The mean age of patients was 43.7 years (30?C63 years). Nodular tumefaction was the most common presenting symptom (6 cases) and inflammatory breast in one case. We found visceral metastases (liver) in one case. Five patients underwent surgery, in association with neoadjuvant chemotherapy in 3 cases and adjuvant chemotherapy in 2 cases. These patients underwent post-operative radiotherapy. Tumor size ranged from 10 to 160 millimeters. We found three tumors with histologic grade II, two tumors with grade III and one tumor with grade I. Lymphatic invasion and lymph node metastases were seen respectively in 3 and 4 cases. Estrogen receptors were expressed in 4/7 cases and progesterone receptors were expressed in 5/7 cases. Her 2 was expressed in 2/7 cases, CK5/6 and EGFR were negative in all cases. The Ki-67 was positive in 3 cases. Four cases have a luminal A profile, one case a luminal B profile, one case Her 2 profile and one case ??normal-breast-like?? profile.

Conclusion

Invasive micropapillary carcinoma of the breast is a rare and aggressive histological variant of breast carcinoma which has a heterogeneous molecular profile; further study is required to confirm these constatations.     

Abdominal Wall Abscess Due to Penetrating Caecal Cancer: An Unusual Presentation

Introduction

The common presentations of caecal cancer are anaemia, weight loss and obstructive symptoms. Caecal cancer perforation and abscess formation are rare and mostly intra- or retroperitoneal.

Case report

We present a case of abdominal wall abscess secondary to penetrating caecal cancer, the inability of computed tomography scan in aiding the diagnosis and successful surgical excision.

Discussion

When caecal cancer presents atypically, the preoperative diagnosis is difficult, but complete excision is possible even with abdominal wall penetration.     

The histone deacetylase inhibitor suberoylanilide hydroxamic acid induces growth inhibition and enhances taxol-induced cell death in breast cancer

Purpose

The histone deacetylase inhibitor (HDACi) suberoylanilide hydroxamic acid (SAHA) enhances taxol-induced antitumor effects against some human cancer cells. The aim of this study is to investigate whether SAHA can enhance taxol-induced cell death against human breast cancer cells and to illustrate the mechanism in detail.

Methods

A panel of eight human breast cancer cell lines and an immortalized human breast epithelial cell line were used to determine the inhibitory effects of SAHA, taxol, or their combination by MTT assay. The effects of SAHA with or without taxol on cell cycle distributions, apoptosis, and protein expressions were also examined. The inhibitory effects on tumor growth were characterized in vivo in BALB/c nude mice bearing a breast cancer xenograft model.

Results

Taxol-resistant and multi-resistant breast cancer cells were as sensitive to SAHA as taxol-sensitive breast cancer cells. A dose-dependent synergistic growth inhibition was found in all the tested breast cancer cell lines treated with the SAHA/taxol combinations. The synergetic effect was also observed in the in vivo xenograft tumor model. The cell cycle analysis and apoptosis assay showed that the synergistic effects resulted from enhanced G2/M arrest and apoptosis.

Conclusions

SAHA increased the anti-tumor effects of taxol in breast cancer in vitro and in vivo. The combination of SAHA and taxol may have therapeutic potential in the treatment of breast cancer.     

A Rare Cause of Distal Bile Duct Stenosis

Introduction

In a patient with a distal bile duct stenosis a definite diagnosis could not be made preoperatively.

Discussion

The histologic evaluation of the surgical resection specimen revealed infiltration of the pancreatic head and the distal bile duct by breast tumor cells.

Conclusion

The metastasis was the only tumor manifestation after mastectomy 12 years ago.     

Differential regulation of sunitinib targets predicts its tumor-type-specific effect on endothelial and/or tumor cell apoptosis

Purpose

Sunitinib is an inhibitor of tyrosine-kinase receptors, and no biomarker predictive of sunitinib response is available. The purpose of this preclinical study was to show whether sunitinib molecular targets could be used as biomarkers to assess tumor response to sunitinib in human cancer cell line xenografts of three different tumor types.

Methods

Using mice xenografted with liver, breast and renal carcinoma cell lines, we sequentially analyzed the effect of 7-day sunitinib treatment on tumor and vascular compartments.

Results

In all xenografts, microvessel damage occurred from Day 1. Tumor damage also occurred in liver, breast, but not in renal xenografts. Using specific human and mouse probes for genes encoding sunitinib targets, we showed a significant relation between apoptotic tumor cell numbers and human PDGFRΒ and RET mRNA expression in liver cancer and to human VEGFR2 expression in breast cancer xenografts. In contrast, in renal cancer xenografts, vascular effect evaluated by measuring endothelial cell apoptosis was related to mouse Vegfr1, Vegfr2 and Vegfa-164 expression.

Conclusion

This study identifies sunitinib vascular and tumor effects according to different tumor types and shows that sunitinib molecular targets used as biomarkers enable assessment of therapeutic response.     

Étude clinicopathologique des carcinomes lobulaires du sein dans le Centre tunisien : à propos de 74 cas

Objective

To analyze the epidemiological characteristics and clinicopathologic features of breast lobular carcinomas in the Central Region of Tunisia.

Patients and methods

A retrospective study was carried out on all breast lobular carcinomas cases diagnosed in the Department of pathology, CHU Farhat Hached Sousse (Tunisia) from 1990 to 2005. The demographic, clinical, histological and treatment were analyzed. The probability of survival or recurrence was calculated using the Kaplan-Meier method. Prognostic factors were studied using the log-rank test.

Results

The mean age was 51 years old and half of the patients were postmenopausal. The average clinical tumor size at diagnosis was 49.9 mm. Seventy five percent of the patients were seen at an advanced stage of the disease (stages T3 an T4). The histological diagnosis was based in all cases on preoperative biopsies. It was in most cases the classical form (94.6%), a low histological grade (64.8%) and hormone receptor positive (65.5%). Surgical treatment was performed in 74.3% cases. Overall survival and disease-free survival at 5 years were respectively 94 and 70.9%. No local recurrence was observed after conservative treatment. Metastasis to lymph node, the clinical stage, the histological grade and non-expression of hormone receptors were significant factors influencing disease-free survival.

Conclusion

Our results were compared with data from literature and show that lobular carcinoma is rare, its diagnosis is often difficult and late, surgical treatment and its prognosis do not currently appear to differ from ductal carcinomas.     

Liposarcome cervical géant exteriorisé et radio-induit : à propos d’une observation

Objective

Study of clinical, radiological and scalable characteristcs of a cervical tumor (liposarcoma), highly malignant, rare and radiation-induced.

Patient and methods

We report the case of a 38 years old patient, with naso-pharynx neoplasy antecedents (UCNT) since 13 years, treated by a curative radiotherapy and an auxiliary chemotherapy, and who has an atypical swelling of malignant pace by its macroscopic aspect and its rapid growth. As exploration, a CT of the neck carried out with an assessment of extension and a surgical removing was the basic treatment.

Results

The CT objectifies a right mass of the neck evoking several etiologies (mesenchymatous tumor, malignant metastatic gonglion ?). The biopsy reveals an atypical liposarcoma, and the assessment of extension returned normaly. We practiced a surgical removing. The histology of the operational part reveals a dedifferenciated liposarcoma. Ablation was not total because of the extension of the tumor to the level of the skull base. Recurrence occurred eight months after.

Conclusion

The LSRI is a rare and late complication of an ionizing treatment. Its aggressiveness depends primarily on the histological form. The treatment is based on surgical ablation.     

Primary amelanotic melanoma of the breast: combating a rare cancer

Background

Malignant melanoma is the foremost cause of metastasis to the breast from extramammary solid neoplasm. However primary melanoma of the breast is a distinct rarity. Primary melanoma involves the skin and less commonly the glandular parenchyma of the breast.

Method

We herein describe a case of primary amelanotic melanoma of the breast parenchyma in a 32-year-old female managed with a combination of surgery, adjuvant radiotherapy and immunotherapy.

Conclusion

This case report aims to increase awareness of unusual neoplasms of the breast which might require a different surgical and adjuvant therapeutic approach.     

The impact of the sentinel node concept on the aesthetic outcome of breast cancer conservative surgery

Background

The sentinel node (SN) concept has brought numerous advantages to women with breast cancer. Sparing axillary node anatomy and physiology may enhance the cosmetic results of breast cancer conservative surgery, either owing to less breast edema or to a better tolerance to the effects of surgery and radiotherapy. Our aim was to compare the cosmetic outcome of two randomized groups of patients, on a subjective and objective basis.

Methods

A consecutive series of breast cancer patients (n = 60) submitted to partial mastectomy plus axillary dissection or partial mastectomy plus SN biopsy (included in a randomized trial) was photographed. Photos were analysed in three ways: by seven different observers according to seven features from poor to excellent; two observers estimated the percentage of breast retraction assessment (pBRA); or by the BCCT.core 1.0^® software.

Results

The panel subjective analysis showed a benefit in terms of the skin colour for the patients submitted to SN biopsy only. This group of patients did not show any advantage in terms of pBRA estimates even after the complex BCCT.core appraisal.

Conclusions

The sentinel node concept is not associated with improvements in the aesthetic outcome of breast cancer conservative treatment.     

Stromal Duodenal Tumor Revealed by an Acute Pancreatitis: Report of a Case

Introduction

Gastrointestinal stromal tumors (GISTs) are one of the most common mesenchymal neoplasms of the human gastrointestinal tract. Duodenal GISTs are very rare and constitute less than 5% of all kind of GISTs.

Case Report

This is a report of a rare gastrointestinal stromal tumor of the duodenum in a 25-year-old female who was presented as an acute pancreatitis.

Discussion

The computed tomography evoked a pancreatic tumor. The patient underwent successful Whipple’s procedure. The histological and immunohistochemical exams of the specimen resection confirmed the diagnosis of duodenal stromal tumor.